TY - JOUR T1 - Serum SP-A as predictor of disease progression in patients with pulmonary alveolar proteinosis JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - P1768 AU - Francesco Bonella AU - Xuan He AU - Shinichiro Ohshimo AU - Josune Guzman AU - Ulrich Costabel Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/P1768.abstract N2 - Background: Surfactant protein A (SP-A) serum levels is known to be increased in pulmonary alveolar proteinosis (PAP),a disorder characterized by alveolar accumulation of surfactant lipoprpoteins. The prognostic value of SP-A in PAP is still unknown and has been investigated in our study.Patients and methods: 28 consecutive patients with PAP were studied prospectively. Serum SP-A was measured by ELISA.We evaluated the correlation between initial SP-A levels, clinical variables and other serum biomarkers. Disease progression was defined as deterioration of symptoms, lung function or chest imaging.Results: The median follow-up time was 510 (90-1890) days. Initial serum SP-A levels correlated inversely with baseline PaO2, FVC und TLCO (r=-0.405, p=0.004; r=-0.46, p=0.001 und r=- 0.462, p=0.003) and directly with AaO2 (r=0.315, p=0.026). Serum SP-A also correlated with LDH (r=0.451, p=0.001) and KL-6 (r=0.363, p=0.008). A correlation between changes in TLCO, PaO2 and AaO2 and changes in serum SP-A during the follow-up was seen (r=-0.7, p=0.002; r=-0.538, p=0.007; r=0.436, p=0.033 respectively). Serum SP-A was higher in patients with disease progression (n=14) (p=0.001). At a cut-off level of 490 ng/mL, serum SP-A predicted disease progression with a sensitivity of 88% and specificity of 75% and the necessity of whole lung lavage (WLL) with a sensitivity of 86% and specificity of 70%. In the multivariate analysis, the initial serum SP-A level was an independent predictor of disease progression (HR 3.9, p=0.046) and of the necessity of WLL (HR 7.2, p=0.003).Conclusions: Serum SP-A appears to have a predictive value for disease progression in PAP. ER -